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Question 1 of 5

1. Question

1 points

A 70 year-old man, who was previously healthy, sustained a stroke in the left side of this structure. Examination reveals dysarthria, Horner’s syndrome, and ipsilateral facial hypalgesia with crossed body hypalgesia. Which other symptom will most likely be present?

Aphasia

Dementia

Intention tremor

Diplopia

Epilepsy

Correct

The structure that has been sketched is the medulla, which should be identified by the paired scalloped, concave nuclei. The lateral medulla contains the cerebellar outflow tracts and several important brainstem nuclei. He has developed the “lateral medullary syndrome.” As part of that syndrome, injury of the cerebellar outflow tract will cause an ipsilateral intention tremor. More important, lesions of the brainstem generally do not cause aphasia, dementia, or epilepsy – all manifestations of cerebral injury.

Incorrect

The structure that has been sketched is the medulla, which should be identified by the paired scalloped, concave nuclei. The lateral medulla contains the cerebellar outflow tracts and several important brainstem nuclei. He has developed the “lateral medullary syndrome.” As part of that syndrome, injury of the cerebellar outflow tract will cause an ipsilateral intention tremor. More important, lesions of the brainstem generally do not cause aphasia, dementia, or epilepsy – all manifestations of cerebral injury.

Question 2 of 5

2. Question

1 points

Regarding genetic diseases that result from excessive trinucleotide repeats, which of the following statements is false?

Juvenile onset Huntington's disease is an example.

Characteristically in a phenomenon "anticipation," successive generations show signs of the disease at an earlier age.

Maternal rather than paternal inheritance confers a greater degree of illness.

Huntington's disease developing in middle-age men is an example.

Correct

Paternal rather than maternal inheritance confers a greater degree of illness. Genetic material in sperm is apparently more unstable than in eggs. Myotonic dystrophy, fragile-X syndrome, certain spinocerebellar ataxias and Huntington’s disease result from expansion of abnormal genes characterized by excessive trinucleotide repeats.

Incorrect

Paternal rather than maternal inheritance confers a greater degree of illness. Genetic material in sperm is apparently more unstable than in eggs. Myotonic dystrophy, fragile-X syndrome, certain spinocerebellar ataxias and Huntington’s disease result from expansion of abnormal genes characterized by excessive trinucleotide repeats.

Question 3 of 5

3. Question

1 points

A 4 year-old mentally retarded boy eats compulsively and voraciously. He eventually develops extreme obesity. Other than poor coordination, his neurologic examination shows no particular abnormality. Also, his routine blood tests, an MRI of the brain, and endocrine tests are normal. His father has mental impairment and obesity, but not to the same extent. At this point, which of the following illnesses is indicated?

Prader-Willi syndrome

Gilles de la Tourette syndrome

Trisomy 21

A hypothalamic tumor

Correct

While all the choices might be considered, the familial incidence, lack of neurologic abnormalities, and normal MRI and endocrine studies eliminate almost all of them. The most likely condition is the Prader-Willi syndrome, which results from abnormal genetic material that is usually inherited from the father.

Incorrect

While all the choices might be considered, the familial incidence, lack of neurologic abnormalities, and normal MRI and endocrine studies eliminate almost all of them. The most likely condition is the Prader-Willi syndrome, which results from abnormal genetic material that is usually inherited from the father.

Question 4 of 5

4. Question

1 points

A 72 year-old man developed dementia. Then he became reticent, but when he spoke his speech was aggressive and contained many sexual innuendos. He was ambulatory and had no focal physical neurologic signs. He had marked frontal release signs and tended to put inedible objects into his mouth. Visuo-spatial relationships are preserved. Which of the following is the most likely diagnosis?

Alzheimer's disease

Frontotemporal dementia

Bovine encephalopathy

Multi-infarct (vascular) dementia

Correct

He has uninhibited behavior, often with a sexual flavor, and other signs of frontal lobe dysfunction. Notably, he has no lateralized signs, but preserved visuo-spatial relationships. This is a case of fronto-temporal dementia, which typically has aspects of the human Kluver-Bucy syndrome. Pick’s disease is probably the commonest cause of this variety of dementia.

Incorrect

He has uninhibited behavior, often with a sexual flavor, and other signs of frontal lobe dysfunction. Notably, he has no lateralized signs, but preserved visuo-spatial relationships. This is a case of fronto-temporal dementia, which typically has aspects of the human Kluver-Bucy syndrome. Pick’s disease is probably the commonest cause of this variety of dementia.

Question 5 of 5

5. Question

1 points

Which of the following visual fields is usually found with nonfluent aphasia?

Correct

Nonfluent aphasia, which usually results from a left cerebral hemisphere lesion, is associated with a right homonymous hemianopsia. Remember that visual fields are portrayed from the patient’s perspective.

Incorrect

Nonfluent aphasia, which usually results from a left cerebral hemisphere lesion, is associated with a right homonymous hemianopsia. Remember that visual fields are portrayed from the patient’s perspective.

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